成人原发免疫性血小板减少症诊治中国专家共识2012修订版解读.pptVIP

概述获得性自身免疫性出血性疾病占出血性疾病1/3，老年人高发以皮肤黏膜出血为主患者可有明显乏力症状

AverageannualITPincidenceby

agegroupandgender(n?=?1145)

LifeQualityinChronicITPPatientsHealthyGeneralPopulationCancerHypertensionArthritisITPITPDiabetesLimoCHF

临床表现出血症状一般与血小板计数负相关部分重度血小板减少患者无出血症状或仅轻度出血老年患者出血发生率明显高于年轻患者注意：乏力与血栓形成

Estimatedannualrateofbleedingaccordingtoagegroup

ITP出血评分量表因素?分值年龄瘀点/瘀斑器官出血≥65岁头面部其它部位鼻衄/牙龈出血/口腔血疱内脏出血（肺、胃肠道、泌尿生殖系统）中枢系统偶发、可自止多发、持续不止不伴贫血伴有贫血危及生命?1√?√??????2?√?√?????3????√√???4??????√??5???????√√王琳侯明.原发免疫性血小板减少症出血评分系统临床应用分析。中华血液学杂志。2012

发病机制对自身抗原免疫耐受缺失血小板生成减少血小板破坏增多

OligoclonalexpansionsofGP-specificCD4+TcellsFogartyPF,etal.ClinAdvHematolOncol2003;1:365-71T-cellactivationinITPSempleetal.Blood1991;78:2619-25Sempleetal.Blood1996;87:4245-54LostofT-celltolerancetoselfantigeninITPPeng，etal.Blood2003；101：2721-26Zhang，etal.JThrombosisHaemostasis2007；6:158–65DisturbedapoptosisofTcellsOlsson,etal.ThrombHaemost2005;93:139-44LossofT-celltolerance

发病机制对自身抗原免疫耐受缺失血小板生成减少血小板破坏增多

PlateletproductionissuboptimalinITPpatientsAutologous111In-plateletstudiesshowplateletproduction≤normalin2/3patientsTPOlevelsnormalin75%ofITPpatients(relativeTPOdeficiency) AutoantibodiesinhibitbothMkgrowthandMkapoptosisTrail-mediatedmegakaryocytepara-apoptosisleadingtoinvitrodysmegakaryocytopoiesisandimpairedplateletproduction

ThrombopoietinlevelsinITPpatients?????????????????????????????????????????????????????????????????????????????????????????????????????????????????????????????????????????????????????????????????????????????Houetal.BrJHaematol1998;101:420-4

DecreasedplateletproductionTrail-mediatedmegakaryocytepara-apoptosisleadingtoinvitrodysmegakaryocytopoiesisplasmaantibodyRemovalofantibodyThenumberofmegakaryocytesMegakaryocyteapoptosisAntibodiesinhibittheGenerationofmegakaryocytesYang,etal.Blood2010;116:4307-16

发病机制对自身抗原免疫耐受缺失血小板生成减少血小板破坏增多

Inc